Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis. Cranial nerve involvement is very rare in CSS. A 59-year-old woman had complained of both hearing impairments for eight months and left facial palsy for three months. Left facial and cochlear neuropathies were detected in electrodiagnostic studies. Paranasal sinus computed tomography (CT) showed chronic pansinusitis. Chest CT revealed eosinophilic infiltration in the right upper lobe. Tissue biopsy of the right inferior turbinate displayed necrotizing vasculitis with eosinophilic infiltration. She was diagnosed as CSS, based on the presence of eosinophilia, pulmonary infiltration, paranasal sinusitis, and biopsy containing blood vessels with extravascular eosinophils. She was treated with intravenous and oral steroids and azathioprine, showing relatively good prognosis on facial palsy and hearing impairment. We report a very rare case of CSS presented with hearing impairment and facial palsy.